Adrenal myelolipomas are rare benign tumors comprising mature adipose tissue and diverse hematopoietic elements. These neoplasms are usually incidental findings, although bulky masses can generate abdominal pain as well as other symptoms related to compression of adjacent organs. Diagnosis is usually provided by ultrasonography or computed tomography. Asymptomatic patients with tumors with a maximum diameter of less than 6cm can benefit from periodic surveillance, whereas local compression symptoms and size larger than 6cm are indications for surgical treatment. We present a case of giant adrenal myelolipoma and provide a review of the literature.